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TAR DNA binding protein of 43 kDa (TDP-43) is a major deposited protein in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitin (FTLD-U).The TDP-43 protein is comprised of an N-terminal random coil, two RNA recognition motifs (RRMs), and a C-terminal glycine rich region (GRR).